Melioidosis is a severe illness caused by Burkholderia pseudomallei, a sepsis-causing bacterial pathogen that is common in East Asia. We reexamined the underlying diagnosis in a 12-year-old boy who was diagnosed in the French West Indies with melioidosis when he was 4 years old. Our investigations led to the determination of chronic granulomatous disease (CGD), an inherited condition characterized by phagocytic cell dysfunction. This is the third reported case of melioidosis associated with CGD to be reported, lending support to an association between the two. The molecular determinants of the susceptibility of CGD patients to B. pseudomallei are still unknown. Our report suggests that CGD should be suspected in children with melioidosis, both in the Americas and probably in other regions world-wide.